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Recent advance in primary immune deficiency disorders

Korean Journal of Pediatrics 2009;52(6):649-654.
Published online June 15, 2009.
Recent advance in primary immune deficiency disorders
Hyoung Jin Kan, Hee Young Shin, Hyo Seop Ahn
Department of Pediatrics, Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea
일차성 면역결핍질환의 최신 지견
강형진, 신희영, 안효섭
서울대학교 의과대학 소아과학교실, 암연구소
Hee Young Shin, Email: hyshin@snu.ac.kr
The immune system is comprised of cells and molecules whose collective and coordinated response to the introduction of foreign substance is referred to as the immune response. Defense against microbes is mediated by the early reaction (innate immunity) and the late response (adaptive immunity). Innate immunity consists of the epithelial barrier, phagocytes, complement and natural killer cells. Adaptive immunity, a more complex defense reaction, consists of activation of later-developed lymphocytes that, when stimulated by exposure to infectious agents, increase in magnitude and defensive capabilities with each successive exposure. In this review we discuss recent advances in important primary immune deficiency disorders of innate immunity (chronic granulomatous disease, leukocyte adhesion deficiency) and adaptive immunity (severe combined immune deficiency, Wiskott- Aldrich syndrome).
Key Words: Primary immune deficiency, Innate immunity, Adaptive immunity

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